Orthostatic headaches

Alok Tyagi

West of Scotland Regional Headache serviceInstitute of Neurological Sciences

Southern General HospitalGlasgow

Orthostatic headaches;Definition

• Headache that comes on shortly after attaining the upright posture and resolves / improves on lying flat.

• A classic orthostatic headache is a post dural puncture headache

• Also seen spontaneously

Overview

• Clinical presentation ; Headache patterns Other neurological symptoms

• Investigations ; MRI

• Management ; Conservative Epidural blood patch

CSF facts

Sites of CSF Production; Choroid Plexus Extra-Choroidal Sources Ependymal Layer Brain Parenchyma Spinal Subarachnoid Space

Sites of CSF absorption; Arachnoid villi Lymphatic drainage Brain capillaries Choroid plexus

Mean CSF volume 157+/-59 ml

Spinal CSF volume 49 +/-12.1 CSF formed at the

rate of 0.35 ml/min

CSF flow

The volume – pressure curve

Steiner LA, Andrews PJ . Br J of Anaesth.2006. 97 (1): 26–38

Monro-Kellie hypothesis

Wolff’s Headache 2007

Headaches due to low CSF pressure

Post dural (post lumbar) puncture headache

CSF fistula headache

Headache due to spontaneous low CSF pressure

Diagnostic criteriaA. Diffuse and/or dull headache that worsens within 15 minutes after sitting or standing, fulfilling criterion D and with 1 of the following:

1. Neck stiffness 2. Tinnitus 3. Hypacusia 4. Photophobia 5. Nausea

B. At least 1 of the following:

1. Evidence of low CSF pressure on MRI (eg, pachymeningeal enhancement) 2. Evidence of CSF leakage on conventional myelography, CT myelography, or cisternography 3. CSF opening pressure 60 mm H2O

C. No history of dural puncture or other cause of CSF fistula

D. Headache resolves within 72 hours after epidural blood patching

Epidemiology

• Prevalance of 1 in 50,000 in the community

• In A/E 5 in 100,000

• Female:Male=1.5:1

• Peak incidence at around 40 yrs of age

Headache patterns in low CSF pressure states

Orthostatic headaches (present when upright, relieved by recumbency)

Orthostatic headaches evolving in months to chronic lingering headaches

Exertional headaches without any orthostatic features

Acute thunder clap–like onset of orthostatic headaches

Second half of the day headaches (often with some orthostatic features)

Other clinical features of low CSF pressure states

Common;

Pain or stiff feeling of neck—sometimes orthostatic

Nausea, sometimes emesis—often orthostatic

Horizontal diplopia (unilateral or bilateral sixth cranial nerve palsy)

Third and fourth cranial nerve palsies (much less common than sixth cranial nerve palsy)

Dizziness

Change in hearing (muffled, distant, distorted, echoed)

Visual blurring

Photophobia

Etiology of low CSF pressure state

Unknown cause (most often) Weakness of the dural sac A. Meningeal diverticula B. Abnormalities of connective tissue

Dural tear from spondylosis or disc herniation

Trivial trauma

Altered distribution of cranio-spinal elasticity (increased compliance of the lumbo-sacral CSF space)

Schievink WI. JAMA. 2006 May 17;295(19):2286-96

Head MRI abnormalities in low CSF pressure states

Diffuse pachymeningeal enhancement

Descent (‘‘sagging’’ or ‘‘sinking’’) of the brain

Descent of cerebellar tonsils (may mimic Type I Chiari)

Obliteration of some of the subarachnoid cisterns (ie, prepontine or perichiasmatic cisterns)

Crowding of the posterior fossa

Enlargement of the pituitary

Flattening or ‘‘tenting’’ of the optic chiasm

Subdural fluid collections (typically hygromas, infrequently hematomas)

Engorged cerebral venous sinuses

Decrease in size of the ventricles (ventricular collapse)

Increase in anteroposterior diameter of the brainstem Mokri B. Neurol Clin. 2004 Feb;22(1):55-74

MRI brain can be normal in up to 20 % of patients with a headache due to a low CSF pressure state. Mokri B. Mayo Clin Proc.1999;74:1113-1123.

Demonstration of a spinal CSF leak

• CT myelogram

• Radionuclide cisternography

• MR imaging

• MR myelography

Myelography

• Early and delayed CT scans need to be performed

• Multiple levels may need to be scanned

• Majority of the leaks are at the cervico-thoracic junction or in the thoracic spine

• Multiple spontaneous CSF leak are common

Mokri B. Neurol Clin. 2004 Feb;22(1):55-74

• MRI myelography can be used to identify the site of CSF leak. Vanopdenbosch LJ et al. J Neurol Neurosurg Psychiatry. 2010 Jun 20 (epub)

Wang YF et al. Neurology. 2009 Dec 1;73(22):1892-8

Spine MRI abnormalities in low CSF pressure states

Extra-arachnoid fluid collections (often extending across several levels)

Extradural extravasation of fluid (extending to paraspinal soft tissues)

Meningeal diverticula

Identification of level of the leak (not uncommonly)

Identification of the actual site of the leak (very uncommonly)

Spinal pachymeningeal enhancement

Engorgement of spinal epidural venous plexus

STIR

T2

Sensitivity of spinal MRI in the diagnosis of low CSF pressure states is 94 % Watanabe et al. Am J Neuroradiol. 2009 Jan: 30(1): 147-152

CSF findings in low CSF pressure states

• The variability is considerable

• CSF pressures are less than 6 cm of water but can be unmeasurable.

• The CSF pressure can be normal

• CSF cell count can be high (upto 100 cells)

• CSF protein can be high (upto 1 gm)

• CSF glucose is never abnormal

Conservative treatment

• CSF leaks can resolve spontaneously and some patients may not require any treatment

• Bed rest

• Treatments with no evidence base; Hydration Abdominal binder Caffeine (oral or iv) Steroids Theophylline

Epidural blood patch (EBP)

• Treatment of choice for patients who fail conservative treatment

• The success rate with each epidural blood patch (when site of CSF leak is unknown) is about 30%

• There is a higher likelihood of long-term (5 year) resolution of SIH symptoms if patients are treated with EBP rather than conservative measures (??)

Possible mechanisms of action of EBP A replacement of CSF volume by blood

An early effect due to volume replacement, a result of dural tamponade

A reduction of CSF absorption probably by restriction of CSF flow

A latent effect probably from sealing of the leak

A reversal of the CSF-blood gradient within the epidural space

A decrease in the compliance of the lower spinal CSF space probably a result of dural stiffening

Schievink WI. Cephalalgia. 2008 Dec;28(12):1345-56

EBP; The procedure

• 10-20 ml blood is injected after which the patient should be placed in a Trendelenburg position for 30-60 minutes

• The injection should be terminated at the first patient complaint of pain, back pressure, or headache

• Complications from an epidural blood patch are backache, neckache, dizziness, auditory disturbances, signs of meningeal irritation, epidural infection, and nerve root compression. Rarely an adhesive arachnoiditis may result

• If the first blood patch fails a large volume patch should be given (20-100 ml)

• An interval of 5 days is recommended between blood patches

Level of EBP

• If the level of the CSF leak is not known a lumbar epidural blood patch should be given first.

• If the level of the CSF leak is not known and a lumbar epidural blood patch has failed a thoraco-lumbar blood patch should be given.

• If the CSF leak is localised to the thoracic level a directed thoracic blood patch should be given

• If the CSF leak is localised to the cervical level a directed cervical blood patch should be given

Management of refractory low CSF pressure states

Continuous epidural saline infusion Epidural infusion of Dextran

Epidural injection of fibrin glue

CSF shunting

Intrathecal fluid infusion

Surgical repairs of the leak

Indications for surgery for low CSF pressure states

• Symptoms severe enough to warrant surgical treatment

• Site of the leak has been identified

• Symptoms refractory to less invasive measures

Mokri B. Cephalalgia 2008 Dec; 28(12); 1345-56

Complications in low CSF pressure states

• Cerebral venous sinus thrombosis Savoiardo M. J Neurol. 2006 Sep;253(9):1197-202

• Subdural haematomas requiring intervention de Noronha RJ. J Neurol Neurosurg Psychiatry. 2003 Jun;74(6):752-5.

• Epidural blood patch related

PrognosisGroup 1 Group2 Group 3 Group 4

Headaches

Onset gradual acute gradual

Severity moderate severe mild moderate

Past history no no yes yes

Other symptoms

IHS criteria yes yes yes yes

Neurological findings no no no yes

MRI Brain abnormal yes (any) yes (subdurals) yes (any) yes (any)

Progress resolved resolved no resolution resolved

Mea et al 2009

Conclusions 1

• Orthostatic headaches are the hallmark presenting feature of low CSF pressure states

• Consider a low CSF pressure state as the cause of a chronic daily headache or a new daily persistent headache

• Ask for an MRI head with contrast if headache is orthostatic

• A significant proportion of low CSF pressure headaches do probably resolve with conservative management / time

• Consider epidural blood patch in patients with low CSF pressure headaches if conservative measures fail

• Milder symptoms of prolonged duration less likely to resolve completely

Conclusions 2